| | Disruption of a Receptor-Mediated Mechanism for Intracellular Sorting of Proinsulin in Familial Hyperproinsulinemia -- ... |
 | | We hypothesize that proinsulin oligomerizes and binds to CPE, a transmembrane sorting receptor, at the TGN via its ELLE sorting signal and enters the immature secretory granule (ISG) subsequent to budding of the TGN. |
 | | Mutant proinsulin that is not bound to CPE at the TGN may enter constitutive secretory vesicles (CV) and be secreted in an unregulated manner. |
 | | Mutant proinsulin that enters ISG by binding to CPE at the TGN or by default (10) would largely be removed into constitutive-like vesicles (CLV) due to inefficient binding to CPE in the ISG, and released primarily unprocessed, but may also include partially processed forms, in an unregulated manner. |
| mend.endojournals.org /cgi/content/full/17/9/1856 (5644 words) |